Iranian Journal of Child Neurology (IJCN)
Volume:7 Issue: 4, Autumn 2013

The approach to a child who has experienced a first unprovoked generalized tonic-clonic seizure is challenging and at the same time controversial. How to establish the diagnosis, ways and means of investigation and whether treatment is appropriate, are different aspects of this subject. In this writing the above mentioned matters are discussed.

Migraine is the most common acute intermittent primary headache in children and prophylactic therapy is indicated in children with frequent or disabling headaches. The purpose of this study was to evaluate the efficacy and safety of topiramate (TPM) for migraine prophylaxis in children. In a quasi-experimental study, monthly frequency, severity and duration of headache, migraine disability, and side-effects were evaluated in 100 children who were referred to the Pediatric Neurology Clinic of Shahid Sadoughi University of Medical Sciences, Yazd, Iran from April 2011 to March 2012, and were treated with 3 mg/kg/day of TPM for three months. Fifty eight (57.4%) girls and 42 (41.6%) boys with the mean age of 10.46±2.11 years were evaluated. Monthly frequency, severity, and duration of headache decreased with treatment from 15.34±7.28 to 6.07±3.16 attacks, from 6.21±1.74 to 3.15±2.22, and from 2.28±1.55 to 0.94±0.35 hours, respectively, and the Pediatric Migraine Disability Assessment score reduced with TPM from 32.48±9.33 to 15.54±6.16. Transient side-effects were seen in 21% of the patients, including hyperthermia in 11%, anorexia and weight loss in 6%, and drowsiness in 4%. No serious side-effects were reported. TPM could be considered as a safe and effective drug in pediatric migraine prophylaxis.

The purpose of this study was to examine the impacts of hinged and solid anklefoot orthoses (AFOs) on standing and walking abilities in children with spastic diplegia. In a quasi-experimental design, 30 children with spastic diplegia, aged 4-6 years were recruited. They were matched in terms of age, IQ, and level of GMFCS E&R. Children were randomly assigned into 3 groups: a hinged AFO group (n=10) plus occupational therapy (OT), a solid AFO group (n=10) plus OT, a control group who used only OT for three months. Gross motor abilities weremeasured using Gross Motor Measure Function (GMFM).We obtained statistically significant differences in the values between baseline and after treatment in all groups. The groups were also significantly different in total GMFM after intervention. Furthermore, there were differences between hinged AFOs and solid AFOs groups, and between hinged AFOs and control groups.We concluded that gross motor function was improved in all groups; however, hinged AFOs group appears to improve the gross motor function better than solid AFOs and control groups.

Febrile seizure is the most common type of seizure in children. Their incidence is 2-5%. There are different hypotheses about relationship between neurotransmitters and trace elements (such as zinc) and febrile seizure. Zinc, as a major element of some enzymes, plays an important role in the central nervous system (CNS) and can affect some inhibitory mechanisms of CNS. The aim of the present study was to determine whether there were any changes in serum zinc level in children with febrile seizure in comparison with febrile children without seizure.This case-control study was performed on 100 patients aged 6 months to 6 years. This study was conducted between January and August 2012, on 50 children with febrile seizures (case) and 50 febrile children without seizures (control), that were referred to Amirkola Children Hospital (a referral hospital in the north of Iran). Two groups were matched for age and sex. The serum zinc levels in the both groups were determined by atomic absorption spectrophotometry method.The mean serum zinc level was 0.585±0.166 mg/L and 0.704±0.179 mg/L in the case group and the control group, respectively (p=0.001). The mean serum zinc level was significantly lower in the febrile seizure group compared to the control groups. Our findings revealed that serum zinc level was significantly lower in children with simple febrile seizure in comparison with febrile children without seizure. It can emphasize the hypothesis that there is a relation between serum zinc level and febrile seizure in children.

Seizure is an emergency in pediatrics. It really matters to the parents of the involved child to have information about the causes, management and prognosis.First unprovoked seizures (FUS) are seizures that occur in patients without fever, trauma or infection. Due to the rapid improvement in diagnostic techniques in the last few decades, the etiology will be revealed and this term will no longer exist. This Study was designed to evaluate brain imaging findings in FUS patients. Ninety-six children with FUS, who were admitted in three major children’s hospitals in Tehran, underwent brain imaging and were enrolled into the study.The decision about the type of imaging (CT or MRI) was based on the patient’s medical and financial conditions. An expert radiologist in the field of pediatric neuroimaging interpreted the images.Altogether, 27.1% had abnormal findings of which 29.2% were in the brain MRI group and 14.3% were in the brain CT scan group.Abnormal results were gliosis (10.4%), hemorrhage (4.2%), dysgenesis (2.1%), dysmyelination (7.3%), encephalomalacy (1%), atrophy (5.2%) and infarction (2.1%). In some patients, the lesions were in 2 or 3 sites and some had more than one type of lesion.There was no association between the duration, age and type of seizure and imaging abnormlities. However, we found an association between the location of the lesion and the type of seizure. We recommend brain imaging in all patients with FUS and apart from some exceptions, brain MRI is superior to CT.

Toxic poisoning with methadone is common in children in Iran. Our study was carried out due to the changing pattern of methadone poisoning in recent years and increasing methadone toxicity. In this descriptive-sectional study, all of the methadone poisoned children younger than 12 years who were admitted to the Loghman Hakim Hospital in 2012, were assessed. Clinical symptoms and signs, para-clinical findings, and treatment were evaluated. In this study, 16 boys and 15 girls who had been poisoned by methadone were enrolled. The mean age of patients was 55 months. All patients had been poisoned randomly or due to parent’s mistakes. The mean time of symptoms onset after methadone consumption was 1 hour and 30 Min, indicating a relatively long time after onset of symptoms.Clinical findings were drowsiness (75%), miotic pupil (68 %), vomiting (61%), rapid shallow breathing (57%) and apnea (40%). In paraclinical tests, respiratory acidosis (69%) and leukocytosis (55.2%) were seen. The most important finding was increase in distance of QT in ECG (23.8%). The mean time of treatment with naloxone infusion was 51 hours. Three percent of patients had a return of symptoms after discontinuation of methadone. In patients with apnea, a longer course of treatment was required, and this difference was significant. Also, 17% of patients with apnea had aspiration pneumonia, which was statistically significant.We suggest long time treatment with naloxone and considering the probability of return of symptoms after discontinuation of methadone.

To assess any relationship between serum sodium changes and seizure occurrence in children aged 2 months to 5 years with hypernatremic dehydration. This cross-sectional study was performed on 63 patients aged 2 months to 5 years from 20 March 2006 to 15 March 2012 at Ghaem Hospital and Dr. Sheikh Hospital in Mashhad, Iran. Patients were divided into 2 groups: case group with hypernatremic dehydration and seizure occurrence, and control group with hypernatremic dehydration and no seizures.The mean age of patients was 10.38 (2-48) months. Thirteen patients had seizures, 11 out of them, before admission and 2 during hospital staying. Serum sodium level at admission in those 2 patients with seizure occurrence after hospitalization was 169 (158-180) mmol/L, and in 50 patients without seizure was 162.8 (148-207) mmol/l. Also, the rate of decrease of serum sodium levels in these 2 cases within the first 12 hours after admission was 1.12, and in those without seizure was 0.54 (mmol/L/hour), and it was 0.47 and 0.53 (mmol/l/hour) after 24 hours of admission, respectively. Severe dehydration was seen in 38.5% of cases and 14% of controls.There was not any relationship between changes in serum sodium level and seizure occurrence in children with hypernatremic dehydration.

Only few Indian reports exist on neuroimaging abnormalities in children with cerebral palsy (CP) from India.We studied the clinico-radiological profile of 98 children diagnosed as CP at a tertiary centre in North India. Relevant investigations were carried out to determine the etiology.Among the 98 children studied, 80.5% were males and 22.2% were premature. History of birth asphyxia was present in 41.9%. Quadriplegic CP was seen in 77.5%, hemiplegic in 11.5%, and diplegic in 10.5%. Other abnormalities were microcephaly (60.5%), epilepsy (42%), visual abnormality (37%), and hearing abnormality (20%). Neuroimaging was abnormal in 94/98 (95.91%).Abnormalities were periventricular white matter abnormalities (34%), deep grey matter abnormalities (47.8%), malformations (11.7%), and miscellaneous lesions (6.4%). Neuroimaging findings did not relate to the presence of birth asphyxia, sex, epilepsy, gestation, type of CP, or microcephaly.Neuroimaging is helpful for etiological diagnosis, especially malformations.

Biotinidase deficiency is one of the rare congenital metabolic disorders with autosomal recessive inheritance. If this disorder is diagnosed in newborn period, could be prevented well from mental and physical developmentaldelay and most of clinical manifestations.The patients were diagnosed as biotinidase deficiency in Neurology Department of Mofid Children’s Hospital in Tehran, Iran, between 2009 and 2012 were included in this study. This study was conducted to define the age, gender, past medical history, developmental status, general appearance, clinical manifestations, neuroimaging findings, and response to treatment in 16 patients with biotinidase deficiency in this department.In clinical presentation, cutaneous lesions were not found in 37% of the patients and 43% patients had not alopecia. 75% patients had abnormal neuroimaging that in 56% of them, generalized brain atrophy and myelination delay were found. Results of the present study showed the efficacy of biotin in early diagnosed patients with seizure and dermatological manifestations. The seizure and skin manifestations were improved after biotin therapy.According to the results of this study, we suggest that early assessment and diagnosis have an important role in the prevention of disease progression and clinical signs.

Kearns-Sayre syndrome is a mitochondrial myopathy, which was first described by Tomas Kearn in 1958. Diagnostic symptoms include retinitis pigmentosa, chronic and progressive external ophthalmoplegia plus one or more of following factors: heart conduction system disorders, cerebellar ataxia, or cerebrospinal fluid (CSF) protein content above 100 mg/dL. The nature of this uncommon disease is yet to be clarified. In this paper, we report a case of Kearns-Sayre syndrome. According to the previous records, the first manifestation of Kearns-Sayre syndrome as hypoparathyroidism is uncommon and in this article, we report a case with this problem.

Episodic ataxia type 2 (EA2) is an inherited autosomal dominant disorder characterized by intermittent ataxia، nausea، vomiting، dysarthria، or nystagmus. We report a case of EA2، which downward gaze palsy exists as a common sign in all her attacks. Responsiveness of EA2 to acetazolamide was observed in this patient.